Pediatric Eisenmenger Syndrome

  • Etiology: Long standing large left to right shunt causing irreversible pulmonary hypertension
    — Initially acyanotic with a left to right shunt
    — Pulmonary arteries thicken and develop pulmonary hypertension
    — Pulmonary pressure becomes greater than systemic pressure
    — Shunt reverses to right to left shunt
    — Deoxygenated right heart blood shunts to systemic circulation resulting in cyanosis
  • Imaging CXR: Prominent central pulmonary artery with periperal pruning of pulmonary blood flow
  • DDX:
  • Complications:
  • Treatment:
  • Clinical:
    — From acyanotic to cyanotic
    — Most commonly caused by ventricular septal defect and less commonly by atrial septal defect and patent ductus arteriosus

Radiology Cases of Eisenmenger Syndrome

Radiology Cases of Eisenmenger Syndrome Due to Ventricular Septal Defect

CXR of Eisenmenger syndrome due to ventricular septal defect / VSD
CXR AP at 4 years of age (above) shows mild prominence of the pulmonary arteries bilaterally. CXR AP at 24 years of age (below) shows marked enlargement of the pulmonary arteries bilaterally with peripheral pruning of the pulmonary vasculature.

Radiology Cases of Eisenmenger Syndrome Due to Ventricular Septal Defect With Septic Pulmonary Emboli

CXR and CT of septic emboli in the lungs / septic pulmonary emboli
CXR AP (above) shows prominence of the central pulmonary arteries and multiple round lesions of varying size throughout both lungs which on the axial CT with contrast of the chest (below) are shown to have indistinct margins.